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BRIEF COMMUNICATION
Year : 2020  |  Volume : 8  |  Issue : 2  |  Page : 72-74

A rare case of unilateral anterior megalophthalmos with developmental glaucoma: Sequelae of megalocornea or a separate entity?


1 Department of Pediatric Ophthalmology and Strabismus, Sadguru Netra Chikitsalaya, Post Graduate Institute of Ophthalmology, Chitrakoot, Madhya Pradesh, India
2 Department of Vitreoretina and Uvea, Sadguru Netra Chikitsalaya, Post Graduate Institute of Ophthalmology, Chitrakoot, Madhya Pradesh, India

Date of Submission21-Jun-2019
Date of Decision18-Sep-2019
Date of Acceptance18-Nov-2019
Date of Web Publication2-Jul-2020

Correspondence Address:
Amit Mohan
Department of Pediatric Ophthalmology and Strabismus, Sadguru Netra Chikitsalaya, Post Graduate Institute of Ophthalmology, Janki Kund, Chitrakoot, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcor.jcor_53_19

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  Abstract 


Megalocornea and megalophthalmos are bilateral developmental anomaly of the anterior segment of the eye. Either of these conditions has a high risk of developing glaucoma. We report the rare case in a 9-year-old child with simple megalocornea in one eye and megalophthalmos in other eye. He has increased corneal diameter in both the eyes and iris stromal hypotrophy exposing the radial vessels, raised intraocular pressure (IOP), and optic nerve head cupping in the left eye (LE). LE trabeculectomy with Ologen was performed. Postoperative IOP was well controlled at 1-year follow-up with favorable bleb morphology.


How to cite this article:
Mohan A, Kumar A, Sen P, Shah C, Sen A, Jain E. A rare case of unilateral anterior megalophthalmos with developmental glaucoma: Sequelae of megalocornea or a separate entity?. J Clin Ophthalmol Res 2020;8:72-4

How to cite this URL:
Mohan A, Kumar A, Sen P, Shah C, Sen A, Jain E. A rare case of unilateral anterior megalophthalmos with developmental glaucoma: Sequelae of megalocornea or a separate entity?. J Clin Ophthalmol Res [serial online] 2020 [cited 2020 Aug 7];8:72-4. Available from: http://www.jcor.in/text.asp?2020/8/2/72/288847



Developmental glaucoma, megalocornea, megalophthalmos Megalocornea is an uncommon developmental anomaly in which the entire anterior segments, including the lens and the zonules, are abnormally enlarged. Megalophthalmos is a similar condition in which, along with megalocornea, there is associated angle anomaly or iris abnormalities. Either of these is bilateral conditions, with high risk of developing glaucoma and cataract. We present the case in a 9-year-old child with megalocornea in one eye and megalophthalmos in the other.


  Case Report Top


A 9-year-old male child presented to our center with complaints of increased size of both eyes since birth, whitish opacity in the left eye (LE) since early childhood, and painless diminution of vision in the LE for 4 months. He was diagnosed with bilateral megalocornea at the age of 3 years in an outside center and was kept on follow-up. However, the child did not comply and came to our center at the age of 9 years with complaints of diminution of vision. One of his maternal uncles has a similar problem of megalocornea.

The affected child was born of second-degree consanguineous marriage. No other siblings had similar complaints. There were no developmental delays in the child. Systemic examination was within the normal limit. At presentation, uncorrected visual acuity was 6/24 in the right eye (RE) and 6/60 in the LE. Corrected distant visual acuity was 6/12 in the RE with − 2.5DC @180° and 6/36 in LE with −2.5DS/0.5DC@120°. [Table 1] shows the anterior segment finding, gonioscopic features, optic disc evaluation, and intraocular pressure (IOP) of both eyes. Corneal diameter in both the eyes was 13 mm in vertical axis and 14 mm in horizontal axis. Anterior segment, disc findings, and IOP were within the normal limit in the RE, whereas in LE, there was anterior corneal mosaic dystrophy, and iris showed anterior stromal thinning with exposed peripheral radial vessels [Figure 1]a and [Figure 1]b. Corrected IOP of the LE was 30 mmHg and gonioscopy showed prominent iris processes in one quadrant. The fundus examination of LE showed an optic cup-to-disc ratio of 0.8:1 with inferior notching [Figure 2]a and [Figure 2]b.
Table 1: Clinical findings of both eyes of child with right eye megalocornea and left eye megalophthalmos

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Figure 1: Anterior segment photograph of a) right eyes showing megalocornea and b) left eye megalocornea with anterior mosaic corneal dystrophy, hypoplastic iris, and prominent radial vessels in the left eye

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Figure 2: Fundus image showing (a) right eye normal cup disc ratio and (b) left eye advanced cupping of disc

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Based on the history and clinical findings, we came to the diagnosis of RE simple megalocornea and LE megalophthalmos with developmental glaucoma. LE trabeculectomy with Ologen was performed. Follow-up was done on postoperative day 1, 1 week, 1 month, 3 months, 6 months, and 1 year. IOP was well controlled on every follow-up. IOP on the last follow-up was 10 mmHg. The bleb was moderately elevated, moderately vascularized with 3 clock hours' extent, and seidels negative on every follow-up [Figure 3].
Figure 3: Postoperative image of bleb

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  Discussion Top


Megalocornea is a developmental anomaly in which there is a nonprogressive bilateral enlargement of the entire anterior segment.[1],[2] Cornea is symmetrically enlarged with diameter of 13–18 mm but with no elevation of IOP.[1],[2],[3] Mode of inheritance is usually X-linked recessive, but autosomal dominant and autosomal recessive linkage is also been described.[2],[3] Arcus lipoides, mosaic corneal dystrophy, pigment dispersion, glaucoma, cataract, and lens dislocation are associated ocular anomalies that manifest in adult life.[2],[3] Families can have some members with megalocornea and others with primary congenital glaucoma. Individuals with megalocornea and their families must therefore be periodically checked for the development of glaucoma. Differentiation between megalocornea and primary infantile glaucoma is difficult. To differentiate, infantile glaucoma is characterized by elevated IOP, breaks in Descemet's membrane, corneal edema, a generally flat iris profile, and less pronounced enlargement of the anterior segment.[4] Posterior positioning of the iris and lens, short vitreous length, and symmetry between the two eye measurements are characteristic of megalocornea.[5]

Anterior megalophthalmos is a rare X-linked recessive disease which manifests as bilaterally symmetrically enlarged clear corneas that remain stable (nonprogressive) throughout life and sometimes contain a stromal mosaic pattern.[6],[7] The iridocorneal angle is open; however, the ciliary body band is wider than the trabecular meshwork and scleral spur on gonioscopy. Iris may manifest a hypoplastic anterior stroma, and transillumination defect may be present.[6],[7],[8],[9] Iridodonesis, miosis, and cataractous lens may be associated findings. The condition predisposes the patient to glaucoma, and hence, the patient should be kept on regular follow-up. The reported incidence of glaucoma is 25% in series of eight cases of anterior megalophthalmos described by Kumawat et al.[10]

Our case was a rare combination in which the child has simple megalocornea in one eye and megalophthalmos with hypoplasia of the iris stroma with exposure of radial blood vessels in the other eye. To our knowledge, no such case has been reported previously after a thorough literature search.

Medical care for patients with megalocornea includes correction of refractive error and thorough evaluation for the findings of anterior megalophthalmos. Regular examination for the development of cataracts and glaucoma is also necessary. Family members should also be screened. Childhood cases of iridogoniodysgenesis respond well to goniotomy or trabeculotomy. Cases with later onset have been managed successfully with medical therapy, argon laser trabeculoplasty, trabeculotomy, or trabeculectomy.[9] In our case, LE trabeculectomy with Ologen was performed. IOP was well controlled in each follow-up with a favorable bleb morphology. The child is still kept on follow-up, and either eye is being regularly monitored. To conclude, every case of megalocornea should be regularly evaluated for glaucoma development, as it may present as developmental glaucoma in later life. Early diagnosis and treatment can be rewarding in these cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Dohlman CH, Larsson S. Megalocornea and cataract. Acta Ophthalmol 1958;36:845-8.  Back to cited text no. 1
    
2.
Berry-Brincat A, Chan TK. Megalocornea and bilateral developmental cataracts. J Cataract Refract Surg 2008;34:168-70.  Back to cited text no. 2
    
3.
Meire FM, Bleeker-Wagemakers EM, Oehler M, Gal A, Delleman JW. X-linked megalocornea. Ocular findings and linkage analysis. Ophthalmic Paediatr Genet 1991;12:153-7.  Back to cited text no. 3
    
4.
Ho CL, Walton DS. Primary megalocornea: Clinical features for differentiation from infantile glaucoma. J Pediatr Ophthalmol Strabismus 2004;41:11-7.  Back to cited text no. 4
    
5.
Meire FM, Delleman JW. Biometry in X linked megalocornea: Pathognomonic findings. Br J Ophthalmol 1994;78:781-5.  Back to cited text no. 5
    
6.
Rao A, Videkar C. Hereditary anterior megalophthalmos with posterior vitreoretinopathy: A surgical challenge. BMJ Case Rep 2014;2014. pii: bcr2013202212.  Back to cited text no. 6
    
7.
Tsai CK, Lai IC, Kuo HK, Teng MC, Fang PC. Anterior megalophthalmos. Chang Gung Med J 2005;28:191-5.  Back to cited text no. 7
    
8.
Kuchenbecker J, Behrens-Baumann W. Ciliary body dysplasia in megalophthalmos anterior diagnosed using ultrasound biomicroscopy. Eye (Lond) 2002;16:638-9.  Back to cited text no. 8
    
9.
Stamper RL, Lieberman MF, Drake MV. Developmental and childhood glaucoma. Becker-Shaffer's Diagnosis and Therapy of the Glaucomas. 8th ed., Ch. 19. China: Mosby Publications; 2009. p. 311-2.  Back to cited text no. 9
    
10.
Kumawat D, Alam T, Sahay P, Chawla R. Ocular abnormalities and complications in anterior megalophthalmos: A case series. Eye (Lond) 2019;33:826-32.  Back to cited text no. 10
    


    Figures

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